Abstract Details

Title Paraneoplastic Cerebellar Degeneration – Uncommon Presentations, Unclassified Autoantibodies

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (8:00 AM-9:00 AM)

Poster/Presentation
Number 9-004

Objective To discuss presentations of paraneoplastic cerebellar degeneration (PCD) with uncommon antibody associations and novel treatment application of onabotulinum-toxin for associated tremor.

Background PCD is a rare neurologic disease, primarily seen in patients with cancer and caused by immune-mediated injury to cerebellar Purkinje cells. Diagnosis relies on classic patterns of cerebellar injury and specific autoantibodies.

Design/Methods Clinical case series

Results Patient A (67-year-old man with squamous cell lung carcinoma) presented with subacute ataxia, gaze-evoked nystagmus, and profound dystonic tremors (null-point, posturing of fingers). MRI brain was negative. Cerebrospinal fluid (CSF) showed lymphocytic pleocytosis and an Acetylcholine Receptor binding antibody, not a previously reported antibody association for PCD. Clinical suspicion of PCD led to treatment with chemoradiotherapy and intravenous immunoglobulin with mild improvement. However, the patient remained with a disabling dystonic tremor. The tremor has been treated with onabotulinum-toxin with complete resolution, the first reported incidence of its use in PCD. Patient B (43-year-old man, no malignancy) presented with ataxia and a down-beating, rotary nystagmus on neck extension. CSF showed elevated protein, four oligoclonal bands (CSF only), and an elevated IgG index, indicative of an immune-based process. MRI brain revealed cerebellar degeneration. Antibody panels reported the presence of an unidentified autoantibody with an immunostaining pattern previously reported among patients with PCD and comorbid seminoma. While no occult malignancy was detected, testicular microlithiasis was found on ultrasound.

Conclusions Rare antibody presentations, and sequela under-score PCD as a still-evolving syndrome. PCD is complex and our understanding of its diverse pathophysiologic underpinnings continues to grow. We described two cases of PCD with uncommon antibodies, underscoring the need for further inclusion of novel antibodies into our repertoire of antibody associations of PCD. Additionally, we described the novel application of onabotulinum-toxin as treatment for dystonic tremors resulting from PCD.

Authors/Disclosures
Susmit Tripathi, MD (New York Presbyterian - Cornell) PRESENTER	Dr. Tripathi has nothing to disclose.
Harini Sarva, MD, FAAN (Weill Cornell Medical Center)	Dr. Sarva has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novo Nordisk. Dr. Sarva has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neuroderm. The institution of Dr. Sarva has received research support from Insightec. The institution of Dr. Sarva has received research support from NeuroNext. The institution of Dr. Sarva has received research support from Neuroderm. The institution of Dr. Sarva has received research support from Sun Pharma. The institution of Dr. Sarva has received research support from Prevail. The institution of Dr. Sarva has received research support from Bluerock Therapeutics. The institution of Dr. Sarva has received research support from Biogen. The institution of Dr. Sarva has received research support from Roche. The institution of Dr. Sarva has received research support from National Institute of Aging. The institution of Dr. Sarva has received research support from Michael J Fox Foundation. The institution of Dr. Sarva has received research support from Novo Nordisk. The institution of Dr. Sarva has received research support from Bukwang. The institution of Dr. Sarva has received research support from Bial. The institution of Dr. Sarva has received research support from Cerevance. The institution of Dr. Sarva has received research support from UCB. The institution of Dr. Sarva has received research support from MeiraGTX.
Rachna Malani, MD (University of Utah)	Dr. Malani has nothing to disclose.

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